A "polymorphous low-grade neuroepithelial tumor of the young (PLNTY)" diagnosed in an adult. Report of a case and review of the literature.
A "polymorphous low-grade neuroepithelial tumor of the young (PLNTY)" diagnosed in an adult. Report of a case and review of the literature.
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DOI：
10.25259/sni_500_2021
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发表时间：
2021
影响因子：
--
通讯作者：
Barbagallo GMV
中科院分区：
文献类型：
--
作者：
Broggi G;Certo F;Altieri R;Caltabiano R;Gessi M;Barbagallo GMV
年轻人的多形性低级别神经上皮肿瘤（PLNTY）是一种罕见的神经病理学实体，近期在神经肿瘤学中被提出。这些肿瘤在组织学上与少突胶质细胞瘤相似，会引发癫痫，发生于儿童和青年人。文献中仅描述了少数PLNTY病例，且所有报道的病例都无一例外地聚焦于儿童和青年人中这些肿瘤的发病情况。
我们报道一例从1岁起就患有癫痫的50岁女性病例。脑部计算机断层扫描和磁共振成像显示存在一个累及左侧颞叶的钙化肿块。肿瘤经手术切除，组织学检查显示为细胞稀少且大量钙化的肿瘤，其形态学和免疫组化特征符合“PLNTY”的诊断。
对文献的回顾显示，文献中报道了31例PLNTY，其中大多数是儿童或青年人。据我们所知，本病例是在中年成人中诊断出的第二例PLNTY，这表明在成人患者中，PLNTY也应始终被纳入低级别脑肿瘤的鉴别诊断中。
Polymorphous low-grade neuroepithelial tumor of the young (PLNTY) is a rare neuropathological entity, recently introduced in neuro-oncology. These tumors, histologically similar to oligodendrogliomas, cause epilepsy, occurring in children and young adults. Only few cases of PLNTY have been described in literature and all reported cases invariably focused on the onset of these tumors in children and young adults. We report the case of a 50-year-old woman suffering from epilepsy since the 1st year of her life. Computed tomography scan and magnetic resonance imaging of the brain documented the presence of a calcified mass involving left temporal lobe. The tumor was surgically excised and the histological examination showed a hypocellular and massively calcified neoplasm with morphological and immunohistochemical features consistent with the diagnosis of “PLNTY.” A review of the literature revealed that there are 31 cases of PLNTY reported in literature, most of which are children or young adults. The present case represents the second PLNTY diagnosed in a middle-aged adult to the best of our knowledge, suggesting that PLNTY should always be included in the differential diagnosis of low-grade brain tumors, also in adult patients.